Neurology: Case of the Month

Test Yourself — Patient 24

Subacute Sclerosing Panencephalitis (SSPE)

  1. Patients with all of the following conditions present with progressive intellectual decline and hepatomegaly, EXCEPT:
    • [ A ] Tay-Sachs disease
    • [ B ] Hurler's syndrome
    • [ C ] Niemann-Pick disease
    • [ D ] Gaucher's disease
  2. All of the following are true statements concerning subacute sclerosing panencephalitis (SSPE), EXCEPT:
    • [ A ] Patients usually present with progressive intellectual decline, myoclonic jerks, and pyramidal and extrapyramidal deficits.
    • [ B ] The presence of elevated rubella antibody titers in the CSF is diagnostic.
    • [ C ] The EEG shows a characteristic abnormality of periodic sharp complexes every 4-14 seconds.
    • [ D ] CNS pathology is nonspecific and consistent with viral encephalitis.
  3. The EEG in SSPE may show all of the following, EXCEPT:
    • [ A ] Normal background activity
    • [ B ] Periodic diphasic sharp wave complexes occurring every eight seconds
    • [ C ] Periodic generalized sharp waves occurring every second
    • [ D ] Diffusely slow background with bifrontal slow activity
  4. Regarding the epidemiology of SSPE, which of the following is FALSE:
    • [ A ] Fifty percent of patients who develop SSPE had measles before two years of age.
    • [ B ] Eighty percent of patients who develop SSPE had measles before four years of age.
    • [ C ] A small number of patients have no clinical history of measles infection.
    • [ D ] Disease occurrence is much greater in females than males.
  5. Which of the following statements concerning SSPE is TRUE:
    • [ A ] Oral Inosiplex and intrathecal alpha interferon are potentially curative.
    • [ B ] Regional variations in the incidence of SSPE most likely result from differences in measles virus load within the various regions.
    • [ C ] The incidence of SSPE is greater in Eastern Europe than in the U.S.
    • [ D ] Eighty percent of patients with SSPE live 10 years or longer following diagnosis.

 

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